This condition is characterized by development of abnormal lymph vessels. Lymph vessels exist in almost every part of your body. That is why GLA can occur anywhere, but the most common locations of the disease are in the bones and lungs. The lesions are caused by an increase in the number of lymph vessels as well as their size.
Some GLAs are associated with a somatic PIK3CA mutation.
Because it is so rare, and commonly misdiagnosed, it is not known exactly how many people are affected by this disease. Generalized lymphatic anomaly (GLA) was formally known as Lymphangiomatosis.
The lymphatic system has three main functions:
- to maintain fluid balance
- to defend the body against disease by producing lymphocytes
- to absorb fats and fat soluble vitamins (A, D, E, and K) from the small intestine and transport them to the blood, bypassing the liver
Bones and organs
The condition may involve a single organ system; 75% of cases involve multiple organs. Although it is technically benign, these disruptive lymphatics tend to invade surrounding tissues and cause problems due to invasion and/or compression of adjacent systems. The condition is most common in the bones and lung and shares some characteristics with Gorham Stout disease. Up to 75% of patients with GLA have bone involvement.
Who has GLA?
GLA can occur at any age, but the incidence is highest in children and teenagers. Signs and symptoms are typically present before the age of 20. The condition is often under-recognized in adults. It strikes males and females of all races and exhibits no inheritance pattern. The medical literature contains case reports from every continent.